Processing and Assembly of the Dystrophin Glycoprotein Complex
نویسندگان
چکیده
منابع مشابه
Dystrophin-glycoprotein complex: post-translational processing and dystroglycan function.
The dystrophin-glycoprotein complex (DGC) is a multimeric transmembrane protein complex first isolated from skeletal muscle membranes (1). The central protein of the DGC is dystroglycan (Fig. 1). In addition to skeletal muscle, dystroglycan is strongly expressed in heart and smooth muscle, as well as many non-muscle tissues including brain and peripheral nerve. In vertebrates, dystroglycan is g...
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The stoichiometry, cellular location, glycosylation, and hydrophobic properties of the components in the dystrophin-glycoprotein complex were examined. The 156, 59, 50, 43, and 35 kd dystrophin-associated proteins each possess unique antigenic determinants, enrich quantitatively with dystrophin, and were localized to the skeletal muscle sarcolemma. The 156, 50, 43, and 35 kd dystrophin-associat...
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Duchenne muscular dystrophy (DMD), the most common inherited neuromuscular disorder, is characterized by progressive muscle wasting and weakness. One third of Duchenne patients suffer a moderate to severe, nonprogressive form of mental retardation. Mutations in the DMD gene are thought to be responsible, with the shorter isoforms of dystrophin implicated in its molecular brain pathogenesis. It ...
متن کاملDifferential requirement for individual sarcoglycans and dystrophin in the assembly and function of the dystrophin-glycoprotein complex.
Sarcoglycan is a multimeric, integral membrane glycoprotein complex that associates with dystrophin. Mutations in individual sarcoglycan subunits have been identified in inherited forms of muscular dystrophy. To evaluate the contributions of sarcoglycan and dystrophin to muscle membrane stability and muscular dystrophy, we compared muscle lacking specific sarcoglycans or dystrophin. Here we rep...
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The purpose of this investigation was to determine the extent to which dystrophin insufficiency caused histomorphological changes in a novel pig model of Becker muscular dystrophy. In our procedures, we used a combination of biochemical approaches, including quantitative PCR and Western blots, along with a histological analysis using standard and immunohistological measures. We found that 8-wk-...
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ژورنال
عنوان ژورنال: Traffic
سال: 2006
ISSN: 1398-9219
DOI: 10.1111/j.1600-0854.2006.00519.x